The Poland syndrome is a rare, sporadic and congenital deformity with uncertain pathogenesis. It is characterized by absence or hypoplasia of the pectoralis major muscle, malformations of the hand and involvement of the ipsilateral mammary gland. A case of a newborn with clinical manifestations suggestive of Poland syndrome on the left hemithorax, associated dextrocardia and defect pulmonary despite being a rare association. Emphasis on the need for a multidisciplinary team in the initial management and long-term treatment.

One case of a 9-year-old male with agenesia of the pectoral muscle is presented. Was submitted because the axilar contour was asymmetric. Clinical examination suggested absence of the right pectoral muscle. CAT confirmed this issue. No other associated malformations or functional deficiencies were noted. As aesthetic impact was light no surgery has been necessary at this moment.

Poland syndrome is a rare congenital anomaly consisting of unilateral partial or total absence of the greater pectoral muscle, associated usually to other malformations in the superior ipsilateral extremities. Its incidence has been estimated to be 1/30,000 live births with a male: female ratio of 3:1, and is mostly right-sided (60-75%). We report the case of a female newborn with a mild defect in the left pectoral muscle, a smaller left areola and hypoplasia of subcutaneous tissue, in association with isolated dextrocardia with the apex pointing to the left. We emphasize the need for additional studies (chest x-ray, bone series, and abdominal ultrasound) in each case.